Jervell and Lange-Nielsen syndrome: neurologic and cardiologic evaluation

Recurrent syncope, malignant ventricular arrhythmias, and sudden death are complications of the long QT syndrome (LQTS). Two well-known syndromes with long QT intervals are known. The Jervell and Lange-Nielsen syndrome (JLNS) is characterized by prolongation of the QT interval, deafness, and autosomal-recessive inheritance, and the Romano-Ward syndrome is characterized by a prolonged QT interval, autosomal-dominant inheritance, and no deafness. In the present study assessment was performed of the diagnostic importance of the ventricular derepolarization parameters, clinical features, and prevalence of JLNS among 132 children with congenital hearing loss (CHL). In the CHL group the mean QT, QTc, JT, and JTc intervals and the dispersion values (QT-d, JT-d, QTc-d, and JTc-d) were significantly longer than those of control subjects (n = 96) (P < 0.05). Patients with CHL and JLNS (n = 5) had significantly longer mean values of QT, QTc, JT, and JTc intervals and dispersion values than those of CHL without JLNS (n = 127) and control subjects (P < 0.05). The results suggest that assessment of ventricular derepolarization parameters in children with CHL will be helpful in the early detection of JLNS because infants with CHL cannot accurately describe the symptoms of syncope.     

Benefit of high-dose methylprednisolone in comparison with conventional-dose prednisolone during remission induction therapy in childhood acute lymphoblastic leukemia for long-term follow-up.

Eight-year event-free survival (EFS) was evaluated in 205 patients with acute lymphoblastic leukemia (ALL), to consider the efficacy of high-dose methylprednisolone (HDMP) given during remission induction chemotherapy between 1 and 29 days. The St Jude Total XI Study protocol was used after some minor modifications in this trial. Patients were randomized into two groups. Group A (n = 108) received conventional dose (60 mg/m(2)/day orally) prednisolone and group B (n = 97) received HDMP (Prednol-L, 900-600 mg/m(2) orally) during remission induction chemotherapy. Complete remission was obtained in 95% of the 205 patients who were followed-up for 11 years; median follow-up was 72 months (range 60-129) and 8-year EFS rate was 60% overall (53% in group A, 66% in group B). The EFS rate of group B was significantly higher than of group A (P = 0.05). The 8-year EFS rate of groups A and B in the high-risk groups was 39% vs 63% (P = 0.002). When we compared 8-year EFS rate in groups A and B in the high-risk subgroup for both ages together /=10 years, it was 44% vs 74%, respectively. Among patients in the high-risk subgroup with a WBC count >/=50 x 10(9)/l, the 8-year EFS was 38% in group A vs58% in group B. During the 11-year follow-up period, a total of 64 relapses occurred in 205 patients. In group A relapses were higher (39%) than in group B (23%) (P = 0.05). These results suggest that HDMP during remission-induction chemotherapy improves the EFS rate significantly for high-risk patients in terms of the chances of cure.     

Recombinant human interleukin-11 prevents mucosal atrophy and bowel shortening in the defunctionalized intestine

BACKGROUND: Mucosal atrophy and bowel shortening are the hallmark of proximal intestinal diversion for extensive necrotizing enterocolitis (NEC) or Thiry-Vella fistulas (TVF), in which the ends of a defunctionalized loop of intestine are exteriorized as stomas. Recombinant human interleukin-11 (rhIL-11) is a pleiotropic cytokine that promotes epithelial regeneration and enhances adaptation after bowel resection. The authors hypothesized that rhIL-11 may prevent mucosal atrophy and bowel shortening in rats with TVF METHODS: After creation of ileal TVF, Sprague-Dawley rats were selected randomly to receive either rhIL-11 or equal volume of 0.1% bovine serum albumin (BSA) subcutaneously daily. On day 14, the TVF were excised and examined morphologically. Enterocyte apoptosis was measured using the TUNEL assay. Mucosal DNA and protein content were measured. RESULTS: Administration of rhIL-11 resulted in a significantly greater weight gain and less shortening of TVF than BSA treatment. TVF from the rhIL-11-treated group showed evidence of hyperplasia and hypertrophy and increased crypt to villus ratio. The BSA group had substantial mucosal atrophy. There was a qualitative decrease in the incidence of apoptosis in the rhIL-11 group. CONCLUSIONS: Recombinant human IL-11 prevents mucosal atrophy and shortening of defunctionalized intestinal loops. It may help reduce the incidence of short gut syndrome in infants with extensive NEC.     

Anaphylactic reaction to polyethylene‐glycol conjugated‐asparaginase: Premedication and desensitization may not be sufficient

In hypersensitive reactions to native L‐asparaginase, either premedication and desensitization or substitution with polyethylene glycol conjugated asparaginase (PEG‐ASP) is preferred. Anaphylaxis with PEG‐ASP is rare. An 8‐year‐old girl and a 2.5‐year‐old boy, both diagnosed as having acute lymphoblastic leukemia, presented with native L‐asparaginase hypersensitivity and substitution with PEG‐ASP was preferred. They received a premedication (methylprednisolone, hydroxyzine and ranitidine) followed by desensitization with PEG‐ASP infusion. Both patients developed anaphylaxis with peg‐asparaginase. These are the first reported cases of anaphylactic reaction to PEG‐ASP, despite the application of both premedication and desensitization. Anaphylaxis with PEG‐ASP is very rare and premedication and desensitization protocols may not prevent these hypersensitive reactions.     

Laser Welding of Ti6Al4V Titanium Alloy in Air and a Water Medium

Ti6Al4V titanium alloys are widely used in a variety of scientific and industrial fields. Laser beam welding is one of the most effective techniques for the joining of titanium plates. The main objective of this study was to investigate the influence of the most important laser parameters on welding performance of titanium alloy in two different physical environments such as air and water (i.e., serum) media. Specifically, the laser beam welding of 2 mm thick Ti6Al4V samples was applied using an Nd:YAG laser in open-air welding using argon as a shielding gas, and in wet welding using a serum environment. The deepest penetration was achieved at −3 mm focal position with 11 J of laser energy in both investigated media (i.e., air and serum). The maximum hardness (1130 HV) was achieved for the focal position of −4 mm in serum medium while it was 795 HV for a focal position of −5 mm in air medium. The minimum (1200 μm and 800 μm) and maximum (1960 μm and 1900 μm) weld widths were observed for air and serum medium, respectively. After the welding process, martensite, massif martensite, and transformed martensite were observed in the microstructure of Ti6Al4V. To the best of our knowledge, the underwater wet welding of titanium alloy was carried out and reported for the first time in this study.     

The Factors Contributing to Death Anxiety in Cancer Patients

Suffering comes in many ways for patients confronting cancer. One of these is an unspecifiable fear about death, which is an existential issue. The aim of this study was to investigate the relationship between death anxiety and its correlates in cancer patients. Seventy cancer patients were assessed using SCID-I, Templer's Death Anxiety Scale, the Hospital Anxiety (A) and Depression (D) Scale, the Distress Thermometer, the Visual Analogue Scale for pain (VAS), the Global Assessment of Functioning, and Glock and Stark's Dimensions of Religious Commitment scales, and these assessments were compared between cancer patients with and without death anxiety. Multiple regression analysis was conducted after correlation analysis between death anxiety and sociodemographic and clinical variables. Axis I psychiatric diagnosis, pain scores, and negative believes about what will happen after death were found to be higher in patients having death anxiety than patients not having death anxiety. Also life expectancy was perceived as shortened in patients with death anxiety. Death anxiety was associated with anxiety, depressive symptoms, and beliefs about what will happen after death. In conclusion, death anxiety could not be regarded as a natural consequence of having cancer; it is associated with the unresolved psychological and physical distress.     

3-Aminobenzamide,a Poly ADP Ribose Polymerase Inhibitor,Attenuates Renal Ischemia/Reperfusion Injury

Introduction. This study was designed to investigate whether 3-amino benzamide (3-AB), a poly (ADP-ribose) polymerase (PARP) inhibitor, has a protective effect on kidney injury induced by renal ischemia/reperfusion (I/R) by decreasing oxidative and nitrosative stress on renal dysfunction and injury. Materials and Methods. Thirty-two male Sprague-Dawley rats were divided into four groups: sham-operated, sham-operated + 3-AB, I/R, I/R + 3-AB. Rats were given 3-AB (100 mg/kg/day ip) 14 days prior to I/R. I/R and I/R + 3-AB groups underwent 60 min of bilateral renal ischemia followed by 6 h of reperfusion. After reperfusion, kidneys and blood were obtained for evaluation. Superoxide dismutase, glutathione peroxidase, malondialdehide, protein carbonyl content, and nitrite/nitrate level (NO_x) were determined in the renal tissue. Serum creatinine (S_Cr), blood urea nitrogen (BUN), and aspartate aminotransferase (AST) were determined in the blood. Additionally, renal sections were used for histological grade of renal injury. Results. 3-AB significantly reduced the I/R-induced increases in S_Cr, BUN, and AST. In addition, 3-AB markedly reduced elevated oxidative stress product, restored decreased antioxidant enzymes, and attenuated histological alterations. Moreover, 3-AB attenuated the tissue NO_x levels, indicating reduced NO production. Conclusions. 3-AB has beneficial effect on renal glomerular and tubular dysfunction in rats' kidneys subjected to I/R injury. Moreover, 3-AB has ameliorating effect on both oxidative stress and nitrosative stress of the kidneys, which correlated with histopathological evaluation.